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With today's carefully designed and staged operations to a Fontan circulation, life expectancy has increased and most children will survive into adult life. The Fontan circulation entails an unphysiological circulation with high risk for multiple organ system dysfunction. Neurodevelopmental disabilities with adverse psychosocial effects are prevalent. The Fontan circulation may eventually fail and necessitate heart transplantation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Fifty years development of the Fontan circulation to today's staged surgical procedures has improved survival but also revealed the burden of a high morbidity for a growing number of patients.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.16023","type":"journal-article","created":{"date-parts":[[2021,7,8]],"date-time":"2021-07-08T07:30:15Z","timestamp":1625729415000},"page":"11-16","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Fontan circulation has improved life expectancy for infants born with complex heart disease over the last 50 years but has also resulted in significant morbidity"],"prefix":"10.1111","volume":"111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3064-155X","authenticated-orcid":false,"given":"Eva","family":"Hedlund","sequence":"first","affiliation":[{"name":"Department of Women's and Children's Health Karolinska Institutet  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo","family":"Lundell","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health Karolinska Institutet  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,7,23]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/thx.26.3.240"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2017.01.057"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(19)40916-1"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5223(19)35174-8"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.athoracsur.2015.07.012"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmjopen-2018-028139"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.3389\/fped.2015.00015"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijcard.2012.03.182"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.118.009145"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/openhrt-2015-000328"},{"key":"e_1_2_11_12_1","first-page":"112","article-title":"Modified Blalock\u2010Taussig shunt. 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